#PediatricVascularDisease #MiddleAorticSyndrome #RenalArteryStenosis #DevelopmentalDisorders #VascularSurgery #OpenSurgery #EndovascularTherapy #Hypertension #KidneyDisease #Rutherford
This summary details developmental disorders affecting the abdominal aorta and renal arteries in children, drawing from Rutherford’s Vascular Surgery. Conditions like Middle Aortic Syndrome (MAS), involving abdominal aortic coarctation, are complex, believed to stem from early fetal developmental issues like improper dorsal aorta fusion. These disorders are often associated with genetic conditions like NF1, which affects over 25% of MAS patients, and can also be linked to inflammatory conditions like Takayasu arteritis or maternal rubella.
A characteristic feature is narrowing (stenosis) of the abdominal aorta, classified by location (suprarenal, interrenal, infrarenal), frequently accompanied by stenosis in the renal arteries (62% of cases) and splanchnic arteries (87%). These narrowings may worsen as a child grows due to disproportionate growth.
The most common clinical sign is severe renovascular hypertension, often difficult to control, potentially causing symptoms like headaches or seizures. Untreated, it can lead to serious complications including chronic kidney disease, stroke, or heart failure. Symptomatic splanchnic narrowing causes postprandial abdominal pain.
Diagnosis typically follows a stepwise imaging approach: ultrasound, then CTA/MRA, with angiography reserved for detailed planning. Medical management focuses on blood pressure control, with careful consideration of medications like ACE inhibitors.
Open surgery is often the preferred treatment for these developmental lesions. Techniques include patch aortoplasty or bypass for aortic coarctation and reimplantation or bypass grafts for renal artery stenosis. Delaying elective surgery until the child is older (age 5-6+) can improve outcomes. Open surgery yields high hypertension cure/improvement rates (often 66-70% cure) with low mortality in experienced centres, though reoperation may be needed later.
Endovascular treatments, while developing, are generally less effective for these specific developmental lesions in children compared to open surgery, showing higher complication and restenosis rates and poorer long-term hypertension outcomes. Failed endovascular intervention can complicate subsequent surgical repair. Stents are usually avoided in growing children. Managing these cases requires a multidisciplinary team.
source
